背景:EB病毒(EBV)感染了全球90%以上的人口。然而,慢性活动性EBV感染(CAEBV)是EBV阳性的T或NK淋巴细胞增生性疾病之一,具有很高的发病率和死亡率。这里,我们报告了一个9岁女孩的CAEBV病例,连续表现为多发性肌炎和冠状动脉扩张(CAD)。
方法:女孩抱怨疲劳超过1个月。肌肉力量检查没有异常发现。血液化学显示丙氨酸转氨酶(ALT)升高,天冬氨酸转氨酶(AST),和肌酸激酶(CK)。磁共振成像(MRI)显示大腿肌肉有斑点高强度信号,肌电图提示肌源性损伤。显著的发现是EBV抗体阳性(EBVEA-IgG,EBVCA-IgG,和EBVNA-IgG),B中EBVDNA拷贝增加,T,和NK细胞,活检肌肉标本中EBV编码的小RNA呈阳性。那个女孩接受了更昔洛韦,静脉注射免疫球蛋白,和甲基强的松龙,她的症状好转了.在住院的第45天,超声心动图显示CAD。她接受了额外的抗凝剂和Tocilizumab。她的病情有所改善,并继续在诊所进行随访,准备进行造血干细胞移植。
结论:这是首例CAEBV合并多发性肌炎和CAD的病例。这种情况使儿童自身免疫性疾病的诊断更加复杂。对于患有非典型多发性肌炎或CAD的儿童,应建议仔细检查隐藏的CAEBV。
BACKGROUND: Epstein-Barr virus (EBV) infects more than 90% of the population worldwide. However, chronic active EBV infection (CAEBV) is one of the EBV-positive T- or NK-lymphoproliferative diseases with high morbidity and mortality. Here, we report a case of a 9-year girl with CAEBV, successively presenting with polymyositis and coronary artery dilation (CAD).
METHODS: The girl complained of fatigue for more than 1 month. Muscle strength examinations had no abnormal findings. Blood chemistries showed elevated alanine aminotransferase (ALT), aspartate aminotransferase (AST), and creatine kinase (CK). Magnetic resonance imaging (MRI) showed spotty high-intensity signals in thigh muscles, and electromyogram suggested myogenic damage. The significant findings were positive EBV antibodies (EBVEA-IgG, EBVCA-IgG, and EBVNA-IgG), increased EBV DNA copies in B, T, and NK cells, and positive EBV-encoded small RNA in biopsy muscle specimen. The girl received ganciclovir, intravenous immunoglobulin, and methylprednisolone, and her symptoms improved. On the 45th day of hospitalization, echocardiograph revealed CAD. She received additional anticoagulants and Tocilizumab. Her condition improved and continued to be followed up at the clinic preparing for hematopoietic stem cell transplantation.
CONCLUSIONS: This is the first reported case of CAEBV successively with polymyositis and CAD. This case makes the diagnoses of autoimmune diseases in children more complicated. Careful investigation of hidden CAEBV should be recommended in children with atypical polymyositis or CAD.